Univentricular Congenital Heart Defects and the Fontan Circulation : Practical Manual for Patient Management

This book provides a concise, practically applicable guide to the management of patients born with a univentricular heart defect. It describes the anatomy and epidemiology of the univentricular heart, while guidance is provided on how to manage both fetal and neonatal patients with this defect, as well as those in later childhood and adulthood. The utilization of the Fontan operation for patients with a univentricular heart is described in detail. Management of early and late Fontan failure is also discussed, and the pregnant patient is also described, enabling the reader to develop a deep understanding of how to manage these patients in their daily practice. Univentricular Congenital Heart Defects: Practical Manual for Patient Management comprehensively reviews the management of univentricular heart defects and the use of Fontan-type surgery. It is a valuable resource for the experienced practitioner seeking a manual on the latest available techniques and for trainees who want to develop a thorough understanding of how to manage patients with these congenital heart defects. Acknowledgements Contents 1: The Univentricular Heart: Past, Present and Future References Part I: Anatomy and Epidemiology of the Functionally Univentricular Heart 2: The Anatomical Substrates of the Univentricular Heart Introduction Solitary Ventricle Defects Amenable to Fontan-Type Palliation Terminology Definition of a Ventricle Hypoplastic Ventricle Classification and Nomenclature of Congenital Heart Disease Tricuspid Atresia HLHS Double Inlet Ventricles Functional Univentricular heart Variants Heterotaxy (Isomerism) Syndromes Pulmonary Atresia References 3: Epidemiology of Univentricular Hearts References Part II: Management of the Fetus and Neonate with a Univentricular Heart 4: Prenatal Diagnosis of the Functionally Univentricular Heart Introduction Prenatal Assessment Morphological Subtypes HLHS and Variants Critical Aortic Stenosis Restrictive Atrial Septum Other Morphological Features Pulmonary Atresia with Intact Ventricular Septum Tricuspid Atresia and Double Inlet Left Ventricle Tricuspid Atresia Double Inlet Left Ventricle Individualised Management Plan Isomerism of the Right and Left Atrial Appendages When Septation of the Heart and a Biventricular Circulation cannot be achieved Effect of Prenatal Diagnosis on Outcome Counselling Prenatal Diagnosis and Neurodevelopmental Outcomes Delivery and Immediate Postnatal Care References 5: Postnatal Management and Pre-Operative Assessment of the Univentricular Patient Postnatal Management Issues Encountered in Postnatal Period Paediatric Cardiology Management Pre-Operative Assessment Confirm the Diagnosis Confirm the Diagnosis—Is it Truly a Univentricular heart Condition? Identify Additional Cardiac Lesions Important Anatomical Features to Consider Include Further Investigations to Aid in Diagnosis Interventions Communication with Parents and Families Summary References 6: Imaging to Aid Decision Making in the Neonate Introduction Morphological Diagnosis Life-maintaining Structures (Fig. 6.1) Procedure-defining Structures Prognostic Structures References 7: Univentricular Heart: Decision Making in the Management of the Neonate Single Ventricle Physiology Arterial Oxygen Saturation in Single Ventricle Physiology Qp/Qs at the Bedside Single Ventricle Anatomy Pre-operative Management Immediate Postnatal Care and Management Pre-operative Care and Management Maximization of Oxygen Delivery to the Tissues Diuretics Continuous Positive Airways Pressure (CPAP) Hemoglobin Threshold for Transfusion Inotropes and Vasoactive Medications Sedation, Muscle Paralysis and Invasive Ventilation Nutrition Surgery Bibliography 8: Catheter Interventions in the Initial Palliation of Univentricular Hearts Introduction Balloon Atrial Septostomy Atrial Septal Stenting Ductal stenting for Duct Dependent Pulmonary Blood Flow Ductal stenting for Duct Dependent Systemic Blood Flow Management of Pulmonary Atresia with Intact Septum Catheter Interventions to Augment Pulmonary Blood Flow in Fallot-Type Lesions with Univentricular Physiology Catheter Interventions After Primary Surgical Palliation Summary References 9: Comfort Care in Patients with a Single Ventricle What Is Paediatric Palliative Care? Why Palliative Care Should Be Offered to Parents of Neonates with a Functional Single Ventricle? Comfort Care Without Surgery Conclusion References Part III: Surgical Management 10: History of the Fontan Surgical Procedure References 11: Early Modifications of Fontan Surgery and Evolution of the Total Cavo-Pulmonary Connection (TCPC) Problems and Modifications of the Atriopulmonary Fontan Lateral tunnel Fontan Extracardiac Conduit Fontan Fenestration Cardiopulmonary Bypass Transcatheter Fontan Y-graft Fontan Powered TCPC References 12: Staged Approach to Total Cavo-Pulmonary Connection Introduction First Stage of Palliation Systemic-to-Pulmonary Artery Shunt Pulmonary Artery Banding Damus-Kaye-Stansel and Norwood Operations Second Stage of Palliation Third Stage of Palliation Fontan Conversion Surgery References 13: Classical Norwood Stage I and Modifications Classical Stage I Norwood Early Modifications Modifications of Aortic Arch Repair Shunt Modifications Single Ventricle Reconstruction Trial References 14: Hybrid Strategies for Hypoplastic Left Heart Syndrome and Comprehensive Stage II Repair Introduction Definitions History Surgical Technique Hybrid Stage I Repair Pulmonary Artery Banding Ductus Arteriosus Stenting and Atrial Septostomy Comprehensive Stage II Repair Outcomes Controversies Future Perspectives References Part IV: The Fontan Physiology 15: Determinants of Cardiac Output and Exercise Tolerance in the Fontan Circulation Introduction Hemodynamic Responses Maladaptive Cardiovascular Responses During Exercise The Role of Skeletal Muscle Mass The High Functioning Fontan Potential Therapeutic Strategies Non-invasive Therapeutic Interventions Drugs Acting on the Ventricular Myocardium Summary References Part V: Follow Up in Childhood of Fontan Patients 16: Neuro-developmental Outcomes of Fontan Patients Introduction Impaired Neurodevelopment in Fontan Physiology Neurodevelopmental Assessment in Fontan Patients Brain MRI Studies (Fontan Only) Acquired Brain Injury Brain Volumetric Injury Neurocognitive Function and Cortical Morphometric and Region-Based Volumetric Injuries Limitations Conclusions References 17: Follow-Up in Childhood of Fontan Patients: Quality of Life Introduction Factors that May Impact HRQoL Why Collecting HRQoL Data Is Important Measuring HRQoL Barriers and Facilitators to Collecting and Using HRQoL Data Moving the Field on: A Strategy for Routine HRQoL Assessment Conclusion References 18: Anticoagulation Strategies in the Single Ventricle Epidemiology and Pathophysiology of Thromboembolic Complications Types of Thromboembolism and Predisposing Factors Managing the Risk of Thromboembolism in Fontan Patients Non-vitamin K Anticoagulants (NOACs) References 19: Electrophysiology Considerations After the Fontan Operation Introduction Sinus Node Dysfunction (SND) Fontan Physiology and the Importance of Sinus Rhythm Scope of the Problem Pacemaker Implantation Surgical Approach Transvenous Approach Intra-atrial Reentrant Tachycardia (IART) Epidemiology Management of Atrial Arrhythmia Sudden Cardiac Death Epidemiology and Risk Factors Prevention of Sudden Cardiac Death Ventricular Dyssynchrony Scope of the Problem Resynchronization Strategies References 20: Managing the Pulmonary Circulation Inefficient Flow Dynamics and Obstruction Pulmonary Vascular Resistance Thrombosis and Embolic Phenomena in the Fontan Circulation Collateral Flow Respiratory Function References 21: Fontan Failure in Children and Cardiac Transplantation Introduction Early Fontan Failure (EFF) Incidence and Pathophysiology of EFF Prevention of EFF: Patient Selection and Preparation for Fontan Management of Early Fontan Failure Late Fontan Failure (LFF) The Chronic Burden of Fontan State Phenotypes of Fontan Failure Management of LFF Cardiac Transplantation for Failing Fontan Circulation in Children Challenges for Transplantation in Failing Fontan Mechanical Cardiac Support (MCS) for Failing Fontan Circulation Outcome of Cardiac transplantation for Fontan Failure in Children References Part VI: Transition, Education and Lifestyle 22: Transition to Adult Life Transitional Care Arrangements Transition Programs of Patients with Congenital Heart Disease Psychological Impact of Chronic Disease in Adult Life Lifestyle Advice Exercise Alcohol/Drugs Piercing, Tattoos and Endocarditis Sexual Health, Contraception and Pregnancy Advice in Fontan Patients Educational and Occupational Status Dental Health Travel with a Fontan Circulation References Part VII: Follow Up in Adult Life 23: Considerations When Developing a Multidisciplinary Adult Fontan Clinic Goals of Multidisciplinary Fontan Programs Benefits of a Multidisciplinary Fontan Clinic Clinic Models Fontan Team Members Pre-clinic Meetings In-clinic flow Frequency of Testing and Subspecialist Care Conclusion References 24: Echocardiography and the Adult Fontan Patient Introduction Fontan Echo Protocol Basic Cardiac Anatomy Type of Fontan Connection and Its Patency Ventricular Systolic and Diastolic Function Atrioventricular Valve Function Systemic Ventricular Outflow Tract Obstruction and Aortic Regurgitation Pulmonary Branches Intracardiac and Extra-cardiac Shunts Other Complications (Table 24.2) References 25: Cardiac MRI Imaging in the Fontan Patient CMR for Surveillance and Prognosis Assessment of Ventricular Function Fontan Pathway Imaging Imaging for Fontan Complications Other MRI Modalities References 26: CT in Patients with Univentricular Hearts and a Fontan Circulation Introduction References 27: Physiological Testing and Basics of Cardiac Catheterisation in Fontan Patients Cardiopulmonary Exercise Testing In Terms of the Cardiovascular Response In Terms of Ventilatory Response Six-Min Walk Test Cardiac Catheterization Indications Preparation and Access Catheters Angiography Calculations and Reporting References 28: Fontan Patients: Psychological Support in Adult Life Introduction Prevalence and Significance of Psychological Distress Psychological Distress and Quality of Life Identification of Psychological Impairment Intervention Pharmacotherapy Psychotherapy Employment and Physical Activity Conclusions References 29: Pregnancy with an Univentricular Heart Introduction Contraception and Fertility Treatment in the Woman with a Fontan Circulation Combined Oral Contraceptive Pill (COCP) Fertility Treatment The Cardiac Obstetric Team Management Plan in the Pregnant Fontan Patient Pre-conception Assessment Maternal and Fetal Risk Models Pregnancy Management Delivery Management Post-partum Management Maternal and Fetal Outcomes Late Effects Following Pregnancy in Fontan Patients Unrepaired Univentricular Hearts Conclusions References 30: Factors Impacting on the Late Outcome of the Fontan Circulation Introduction Era of Surgical Intervention Down Syndrome Low Birthweight Heterotaxy Syndrome Type of Fontan Procedure Performed Ten Commandments Ventricular Morphology Pulmonary Artery Size Catheter-Based Interventions Quality of Life Outcomes References Part VIII: A Multi-system Disorder 31: Electrophysiology Considerations and Management of Arrhythmias After the Fontan Operation Introduction Types of Arrhythmia The Effects of Atrial Arrhythmias in Fontan Patients Arrhythmia Substrates in Univentricular Hearts After Surgery Arrhythmia After “Classical” Atriopulmonary Fontan Operations Arrhythmia After TCPC Conversion Arrhythmia in Heterotaxy Syndrome (Isomerism) Pharmacological Options Non-pharmacologic Arrhythmia Management Options Prerequisites for Invasive EP Procedures Remote Magnetic Navigation in Difficult Access Cases High-Resolution Mapping to Better Delineate the arrhythmia Substrate Sinus Node Dysfunction (SND) Fontan Physiology and the Importance of Sinus Rhythm Scope of the Problem Pacemaker Implantation Sudden Cardiac Death Epidemiology and Risk Factors Prevention of Sudden Cardiac Death Ventricular Dyssynchrony Scope of the Problem Resynchronization Strategies References 32: A Multi-system Disorder: Extracardiac Complications Introduction Fontan Associated Liver Disease Kidney Function The Lymphatic System The Respiratory System Cognitive Function Psychological Health Musculoskeletal Health and Body Composition The Immune System The Haematological System References Part IX: The Failing Adult Fontan Patient 33: The Failing Adult Fontan Patient Pathophysiology Concept of Fontan Failure Determinants of Cardiac Output and Exercise Intolerance The Estimation and Importance of a Low Pulmonary Vascular Resistance Assessment of “Failing Fontan” Patients Diastolic and Systolic Dysfunction of the Systemic Ventricle Chronotropic Response Causes of Fontan Failure Presentation and Assessment of the Patient with Fontan Failure The Medical History and Symptoms of Fontan Failure Signs of Fontan Failure Extra-cardiac Involvement in Fontan Failure Clinical Work-Up of the Patient with Fontan Failure Medical Management Heart Failure Therapy Pulmonary Vasodilator Therapy Medical Therapy for Extra-cardiac Features of Fontan Failure References 34: Catheter-Based Interventions in Adult Life for the Failing Adult Fontan Patient Introduction Reasons to Intervene in the Failing Adult Fontan Patient Relieve Obstruction Prevent Overcirculation Improve Cardiac Output and Reduce Systemic Venous Pressure Create or Redirect Pulmonary Blood Flow (PBF) Relieve Cyanosis Address Valvular Dysfunction Prevent Stroke Treat Complications Specific to the Fontan Circulation Prepare for Transplant Interventions in the Failing Adult Fontan Patient Balloon Angioplasty/Stent in the Fontan Circuit, Branch Pulmonary Arteries, or Residual Coarctation of the Aorta Occlusion of Veno-Venous Collaterals Occlusion of Arteriopulmonary Collaterals Coiling Pulmonary Macro-Arteriovenous Malformations Creation of a Fenestration Closure of a Fenestration Percutaneous Lymphatic Embolization Transcatheter Valve Replacement/Repair References 35: Surgical Interventions in Adult Fontan and Transplantation Introduction Surgical Interventions to Optimize the Fontan Circulation General Principles Cardiac Arrhythmias and Pacemaker Therapy Surgery for Atrioventricular Valve Failure Optimization of the Fontan Pathway Systemic Outflow Tract Intervention Lymphatic System Interventions Fontan Conversion Transplantation Mechanical Circulatory Support (MCS) References 36: Palliative Care Strategies in Adult Patients Introduction Terminology and the Concept of Advance Care Planning Initiation of Advance Care Planning Discussions in Fontan Patients Practical Recommendations on ACP and Effective Communication Regarding ACP Special Considerations of Palliative Care Management in Fontan Patients Palliative and End-of-Life Care Strategies (Modified and Reprinted with Permission [19]) References 37: Role of Mechanical Support for Failing Single Ventricle References 38: Pathological Aspects of Post-Fontan Procedures Heart Transplantation and Heart and Liver Transplantation References Index